Gorlin-Goltz syndrome.

BACKGROUND: Gorlin-Goltz syndrome is an autosomal dominant inherited disorder characterized by a predisposition to various cancers. Clinicopathological findings of syndrome are very diverse and many symptoms begin to manifest in a certain period of life. CASE: The authors describe a case report of a man who, at the age of 34 years, presented to a dermatologist with multiple tumor lesions of the skin. The lesions started to develop when he was 30 years old and thereafter increased in number. Histology revealed superficial, superficial-nodular and nodular basal cell carcinomas. A total of 11 basal cell carcinomas were surgically removed and microscopically investigated. The others were treated locally with imiquimod cream and cryotherapy. In addition, he was found to have multiple odontogenic keratocysts in the jaw and mandible, as well as supernumerary and retinated teeth. Stomatologic and maxillofacial surgery interventions were performed. Further clinical and imaging examinations confirmed macrocephaly, hypertelorism, calcification of falx cerebri, and abnormalities of the cervical vertebrae. The spectrum of pathological findings met the diagnostic criteria of Gorlin-Goltz syndrome. CONCLUSION: Although Gorlin-Goltz syndrome is very rare in routine practice, it usually represents a serious disease with multiple organ system involvement. From a prognostic point of view, early diagnosis with adequate therapy is critical. If a diagnosis is confirmed, lifetime dispensary care with interdisciplinary medical cooperation is necessary. The authors would like to thank all physicians who participated in the diagnostics and therapy of the presented patient. The authors declare they have no potential confl icts of interest concerning drugs, products, or services used in the study. The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers. Submitted: 30. 8. 2018 Accepted: 8. 1. 2019.

Basal Cell Carcinoma Multiplicity - a Retrospective Analysis of 899 Biopsy-proven Patients from a Single Institute.

BACKGROUND: An interesting clinical feature of basal cell carcinoma (BCC) of the skin is a marked interpatient variation in tumor number, lesion accrual and anatomic distribution. We analyzed a proportion of patients with multiple BCCs in the cohort of pathology report-confirmed cases of BCC and investigated clinicopathological differences between individuals suffering from multiple tumor lesions and patients with a single tumor. MATERIAL AND METHODS: All consecutive patients with primary cutaneous BCCs, who were histologically diagnosed at our Department of Pathology during a 10-year period were enrolled into the study. RESULTS: A cohort of 899 participants with a total of 1,239 histologically proven primary BCCs were assessed. Of them, 728 (81%) had single BCC and 171 (19%) had multiple BCCs. Multiple lesions occurred more frequently in men than women. Mean number of tumors per patient was 1.5 in males and 1.2 in females. Among participants with multiple BCC manifestation, there was a steady increase of the male-to-female ratio with rising tumor number per individual. In the multiple BCC subgroup, the tumors were found more commonly in the trunk and upper limbs, and less frequently in the face. Histologically, these BCCs much more commonly included superficial subtype. There was a positive correlation between the non-aggressive histologic phenotype of BCC and multiple tumor presentations on the one hand, and the aggressive histologic phenotype of BCC and a single tumor occurrence on the other. CONCLUSION: Our analysis shows that clinicopathological features associated with multiple BCC manifestations include male gender, tumor location in the trunk and upper extremities, and superficial histological subtype. Focus on this risk profile may be beneficial for clinical screening and may help clinicians in the selection of individuals, who should be followed-up more closely.Key words: basal cell carcinoma - single and multiple manifestations - clinicopathological differences.

Fibroepithelioma of Pinkus.

Basal cell carcinoma (BCC) is recently the most common cancer in humans characterized by several histopathological subtypes. Fibroepithelioma of Pinkus (FEP) is traditionally classified as a very rare variant of BCC, however, it manifests clinical and morphological differences that distinguish it from most other types BCCs. This study was performed to evaluate the incidence of FEP and clinical-pathological characteristics of patients diagnosed with this tumor. Four cases of primary FEP (3 females, 1 male, mean age 53.4 y) were analyzed retrospectively. The prevalence of FEP was 0.7 % of all diagnosed BCCs. Topographically, tumors were localized on the right brachium, right gluteal region, left mesogastrium, and right side of the abdomen. Histological examination showed typical anastomosing cords of basaloid cells extending from the overlying epidermis into the loose fibrous stroma in the dermis. Mitotic activity or significant cellular atypia, as well as sign of solar dermatosis were absent. Mean horizontal and vertical diameter of the lesions were 7.7 and 2.8 mm, retrospectively. We did not observe infiltration of deeper skin structures. All lesions were removed completely and classified as pathological stage pT1. Three cases manifested typical picture of a "pure" FEP, one lesion had partially a feature of nodular type BCC, too. Although FEP is conventionally considered as indolent BCC variant with a favorable clinical outcome, recent evidences also favors its classification as a form of trichoblastoma. This is appropriate example how some types of cutaneous tumors can overlap in terms of dignity and clinical-morphological characteristics. This should be taken into account in differential diagnosis and in predicting of biological behaviour of the individual tumors of the skin (Tab. 2, Fig. 3, Ref. 26).

Comparision of histological types of primary and subsequent relapsing basal cell carcinomas of the skin.

BACKGROUND: Basal cell carcinoma (BCC) recurrences are relatively frequent event in a routine dermatologic practice. One of the most important factor which impacts risk of their development is a histomorphological appearance of tumor. DESIGN: The purpose of our study was to compare histological types of primary and corresponding relapsing BCCs of the skin. MATERIAL AND METHODS: The study included 36 cases of BCC recurrences from 34 patients, 17 women and 17 men. The patients ranged in age from 32 to 97 years, with a mean age of 67.1 years at the time of (the first) recurrence. RESULTS: Both tumor groups generally exhibited the same proportion of indolent and aggressive histological phenotype. In 21 cases (58.4%), we found an identical histological BCC type in primary and subsequent relapsing lesion. In 3 cases (8.3%), primary lesion showed indolent histological features without aggressive--growth component, while recurrent tumor already manifested it. Conversely, in next 3 cases (8.3%) primary tumor exhibited focal infiltrative-growth features and corresponding relapsing lesion did not. Of the remaining 9 cases (25%), histomorphological phenotype was not identical, but it showed the same prognostic histological tumor variant. CONCLUSION: Based on the results of our study it can be assumed that a BCC recurrence is a dynamic histogenetic process, during which the phenotypic transformation and the changes in histomorphological picture of lesions occur, probably as a result of the interactions between cancer cells and re-modulated surrounding stroma.

[Basal cell carcinoma of the skin--biological behaviour of the tumor and a review of the most important molecular predictors of disease progression in pathological practice]. / Bazocelulárny karcinóm koze--biologické správanie nádoru a prehl'ad najvýznamnejsích molekulových ukazovatel'ov progresie ochorenia v praxi patológa.

Basal cell carcinoma of the skin is currently the most frequent malignancy in human population. Basal cell carcinoma represents a heterogeneous group of tumors with a variable clinical and morphological picture. Based on its biological behaviour, we generally differentiate between indolent (superficial and nodular) and aggressive type (infiltrative, micronodular, and metatypical) of basal cell carcinoma. Because of the different biological characteristics of these tumors, it is questionable whether they are a part of a continuous spectrum of carcinogenesis, starting with indolent and ending with aggressive forms, or they represent separate developmental lines. In the current clinical practice, there is an increasing demand for identification of tumors that are prognostically more adverse and their impact on the overall health status of patients is more serious. Recent advances in pathology and molecular medicine allow identification of various biomarkers from tumor tissue that are significantly involved in the mechanisms of malignant cell transformation. Detection of these biomarkers is of great importance in predicting further clinical behaviour of the cancer. The authors of the paper present basic information about biological behaviour of cutaneous basal cell carcinoma and provide an overview of the most important biomarkers that influence the clinical outcome and disease progression and are detectable through a routine biopsy tissue examination. It is now necessary to search for novel histological and molecular parameters that, in the future, could have a prognostic value in diagnostic and therapeutic process of this disorder.